以皮肤瘀斑为首发症状的母细胞性浆细胞样树突细胞肿瘤2例及临床病理分析  

Report and Clinicopathological Analysis of Two Cases of Blastic Plasmacytoid Dendritic Cell Neoplasm with Skin Ecchymosis as the First Symptom

作　　者：万红萍[1] 盛以芸[1] 余克涵[1] 熊振芳[1] 涂露霞[1] 陶雪勤[1] 

机构地区：[1]南昌大学第一附属医院病理科,江西南昌330006

出　　处：《中国皮肤性病学杂志》2018年第2期170-174,共5页The Chinese Journal of Dermatovenereology

摘　　要：目的报告2例母细胞性浆细胞样树突细胞肿瘤(BPDCN),并进行分析。方法对南昌大学第一附属医院病理科2007年1月-2017年4月收治2例BPDCN进行回顾分析,其中1例为误诊病例。结合文献探讨其临床病理特点。结果两例均为成年女性,以皮肤瘀斑为首发症状就诊。组织病理表现为形态单一的中等大小肿瘤细胞弥漫密集分布于真皮及皮下脂肪组织间,但不侵犯表皮。免疫组化示瘤细胞表达CD123、CD4、CD56、CD43,但CD20、CD3、CD8、TIA-1、粒酶B、Td T、MPO、CD68等均不表达,EB病毒编码RNA(EBER)检测阴性。结论 BPDCN是罕见的淋巴造血系统恶性肿瘤,预后差。常以无特异性的皮肤损害开始,易造成误诊。早期正确诊断极为重要。Objective Two cases of blastic plasmacytoid dendritic neoplasm（BPDCN）were reported and analyzed.Methods Two cases of BPDCN were admitted by the department of pathology in the First Affiliated Hospital of Nanchang University from January 2007 to April 2017 were analyzed retrospectively.One of them was misdiagnosed.The clinicopathological characteristics were discussed and the relevant literatures were reviewed as well.Results Both patients were female adult with skin ecchymosis as the first symptom.The histopathological examination showed that the intermediate-sized cells infiltrated diffusely into the dermis and subcutaneous adipose tissue,but no tumor cell presented in epidermis.Immunohistochemistry stain showed that CD123,CD4,CD56,CD43 were positive and that CD20,CD3,CD8,TIA-1,GranzymeB,TdT,MPO,CD68 and EBER were negative.Conclusion BPDCN is a rare malignant tumor of the lymphatic hematopoietic system and has a poor prognosis.It often starts with nonspecific skin lesions and is prone to misdiagnosis.Early correct diagnosis is of great importance.

关 键 词：母细胞性浆细胞样树突细胞肿瘤 皮肤瘀斑 免疫组织化学 

分 类 号：R739.5[医药卫生—肿瘤]